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A Biochemical Investigation

Arthur L. Prensky, MD; Sheila Carr, AB; Hugo W. Moser, MD

Arch Neurol. 1968;19(6):552-558.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

IN 1950, Alvord et al¹ first suggested that the principal neuropathological feature of phenylketonuria (PKU) was a delay in the development of myelin. Subsequently, other investigators described areas of pallor in sections of central white matter stained for myelin and retarded myelination of the long tracts.^2,3 Similar abnormalities were found in maple syrup urine disease (MSUD).^4-6 Biochemical investigations of the cerebral white matter of patients with these diseases have also indicated that myelin was accumulating at a slower rate than that found in normal brains. In both PKU and MSUD, the water content of cerebral white matter was increased and the lipid content reduced.^7-10 The reduction of cerebrosides exceeded that of other lipid classes,^9-11 which is to be expected, since the accumulation of this lipid most closely parallels the formation of normal myelin.¹²

While biochemical studies of this type confirmed prior histological observations, . . . [Full Text PDF of this Article]

Author Affiliations
Boston

From the Department of Neurology, Harvard Medical School, Cambridge, Mass, the Joseph P. Kennedy, Jr., Memorial Laboratories and the Neurology Service at the Massachusetts General Hospital, Boston, and the Walter E. Fernald State School, Waltham, Mass. Dr. Prensky is currently at the St. Louis Children's Hospital, St. Louis.

Footnotes
Submitted for publication May 25, 1968; accepted Aug 5.

Reprint requests to Massachusetts General Hospital, Boston 02114 (Dr. Moser).

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